Panniculitis in childhood-onset systemic lupus erythematosus: a multicentric cohort study

Abstract Objective To evaluate prevalence, clinical manifestations, laboratory abnormalities, treatment and outcome in a multicenter cohort of childhood-onset systemic lupus erythematosus (cSLE) patients with and without panniculitis. Methods Panniculitis was diagnosed due to painful subcutaneous no...

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Main Authors: Mônica Verdier, Pedro Anuardo, Natali Weniger Spelling Gormezano, Ricardo Romiti, Lucia Maria Arruda Campos, Nadia Emi Aikawa, Rosa Maria Rodrigues Pereira, Maria Teresa Terreri, Claudia Saad Magalhães, Juliana C. O. A. Ferreira, Marco Felipe Castro Silva, Mariana Ferriani, Ana Paula Sakamoto, Virginia Paes Leme Ferriani, Maraísa Centeville, Juliana Sato, Maria Carolina Santos, Eloisa Bonfá, Clovis Artur Silva
Format: Article
Language:English
Published: BMC 2019-01-01
Series:Advances in Rheumatology
Subjects:
Online Access:http://link.springer.com/article/10.1186/s42358-019-0049-9
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author Mônica Verdier
Pedro Anuardo
Natali Weniger Spelling Gormezano
Ricardo Romiti
Lucia Maria Arruda Campos
Nadia Emi Aikawa
Rosa Maria Rodrigues Pereira
Maria Teresa Terreri
Claudia Saad Magalhães
Juliana C. O. A. Ferreira
Marco Felipe Castro Silva
Mariana Ferriani
Ana Paula Sakamoto
Virginia Paes Leme Ferriani
Maraísa Centeville
Juliana Sato
Maria Carolina Santos
Eloisa Bonfá
Clovis Artur Silva
author_facet Mônica Verdier
Pedro Anuardo
Natali Weniger Spelling Gormezano
Ricardo Romiti
Lucia Maria Arruda Campos
Nadia Emi Aikawa
Rosa Maria Rodrigues Pereira
Maria Teresa Terreri
Claudia Saad Magalhães
Juliana C. O. A. Ferreira
Marco Felipe Castro Silva
Mariana Ferriani
Ana Paula Sakamoto
Virginia Paes Leme Ferriani
Maraísa Centeville
Juliana Sato
Maria Carolina Santos
Eloisa Bonfá
Clovis Artur Silva
author_sort Mônica Verdier
collection DOAJ
description Abstract Objective To evaluate prevalence, clinical manifestations, laboratory abnormalities, treatment and outcome in a multicenter cohort of childhood-onset systemic lupus erythematosus (cSLE) patients with and without panniculitis. Methods Panniculitis was diagnosed due to painful subcutaneous nodules and/or plaques in deep dermis/subcutaneous tissues and lobular/mixed panniculitis with lymphocytic lobular inflammatory infiltrate in skin biopsy. Statistical analysis was performed using Bonferroni correction(p < 0.004). Results Panniculitis was observed in 6/847(0.7%) cSLE. Painful subcutaneous erythematosus and indurated nodules were observed in 6/6 panniculitis patients and painful subcutaneous plaques in 4/6. Generalized distribution was evidenced in 3/6 and localized in upper limbs in 2/6 and face in 1/6. Cutaneous hyperpigmentation and/or cutaneous atrophy occurred in 5/6. Histopathology features showed lobular panniculitis without vasculitis in 5/6(one of them had concomitant obliterative vasculopathy due to antiphospholipid syndrome) and panniculitis with vasculitis in 1/6. Comparison between cSLE with panniculitis and 60 cSLE without panniculitis with same disease duration [2.75(0–11.4) vs. 2.83(0–11.8) years,p = 0.297], showed higher frequencies of constitutional involvement (67% vs. 10%,p = 0.003) and leukopenia (67% vs. 7%,p = 0.002). Cutaneous atrophy and hyperpigmentation occurred in 83% of patients. Conclusions Panniculitis is a rare skin manifestation of cSLE occurring in the first three years of disease with considerable sequelae. The majority of patients have concomitant mild lupus manifestations.
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spelling doaj.art-1630c38151924a9cb057e616d43a42902022-12-22T00:16:00ZengBMCAdvances in Rheumatology2523-31062019-01-015911510.1186/s42358-019-0049-9Panniculitis in childhood-onset systemic lupus erythematosus: a multicentric cohort studyMônica Verdier0Pedro Anuardo1Natali Weniger Spelling Gormezano2Ricardo Romiti3Lucia Maria Arruda Campos4Nadia Emi Aikawa5Rosa Maria Rodrigues Pereira6Maria Teresa Terreri7Claudia Saad Magalhães8Juliana C. O. A. Ferreira9Marco Felipe Castro Silva10Mariana Ferriani11Ana Paula Sakamoto12Virginia Paes Leme Ferriani13Maraísa Centeville14Juliana Sato15Maria Carolina Santos16Eloisa Bonfá17Clovis Artur Silva18Pediatric Rheumatology Unit, Children’s Institute, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao PauloPediatric Rheumatology Unit, Children’s Institute, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao PauloPediatric Rheumatology Unit, Children’s Institute, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao PauloDivision of Dermatology, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao PauloPediatric Rheumatology Unit, Children’s Institute, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao PauloDivision of Rheumatology, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao PauloDivision of Rheumatology, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao PauloPediatric Rheumatology Unit, Universidade Federal de São PauloSão Paulo State University (UNESP) – Faculdade de Medicina de BotucatuPediatric Rheumatology Unit, Children’s Institute, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao PauloPediatric Rheumatology Unit, Children’s Institute, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao PauloPediatric Rheumatology Unit, Children’s Institute, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao PauloPediatric Rheumatology Unit, Universidade Federal de São PauloPediatric Rheumatology Unit, Ribeirão Preto Medical School – University of São PauloSão Paulo State University of Campinas (UNICAMP)São Paulo State University (UNESP) – Faculdade de Medicina de BotucatuIrmandade da Santa Casa de Misericórdia de São PauloDivision of Rheumatology, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao PauloPediatric Rheumatology Unit, Children’s Institute, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao PauloAbstract Objective To evaluate prevalence, clinical manifestations, laboratory abnormalities, treatment and outcome in a multicenter cohort of childhood-onset systemic lupus erythematosus (cSLE) patients with and without panniculitis. Methods Panniculitis was diagnosed due to painful subcutaneous nodules and/or plaques in deep dermis/subcutaneous tissues and lobular/mixed panniculitis with lymphocytic lobular inflammatory infiltrate in skin biopsy. Statistical analysis was performed using Bonferroni correction(p < 0.004). Results Panniculitis was observed in 6/847(0.7%) cSLE. Painful subcutaneous erythematosus and indurated nodules were observed in 6/6 panniculitis patients and painful subcutaneous plaques in 4/6. Generalized distribution was evidenced in 3/6 and localized in upper limbs in 2/6 and face in 1/6. Cutaneous hyperpigmentation and/or cutaneous atrophy occurred in 5/6. Histopathology features showed lobular panniculitis without vasculitis in 5/6(one of them had concomitant obliterative vasculopathy due to antiphospholipid syndrome) and panniculitis with vasculitis in 1/6. Comparison between cSLE with panniculitis and 60 cSLE without panniculitis with same disease duration [2.75(0–11.4) vs. 2.83(0–11.8) years,p = 0.297], showed higher frequencies of constitutional involvement (67% vs. 10%,p = 0.003) and leukopenia (67% vs. 7%,p = 0.002). Cutaneous atrophy and hyperpigmentation occurred in 83% of patients. Conclusions Panniculitis is a rare skin manifestation of cSLE occurring in the first three years of disease with considerable sequelae. The majority of patients have concomitant mild lupus manifestations.http://link.springer.com/article/10.1186/s42358-019-0049-9Lupus erythematosus panniculitsChildhoodSystemic lupus erythematosus and multicenter study
spellingShingle Mônica Verdier
Pedro Anuardo
Natali Weniger Spelling Gormezano
Ricardo Romiti
Lucia Maria Arruda Campos
Nadia Emi Aikawa
Rosa Maria Rodrigues Pereira
Maria Teresa Terreri
Claudia Saad Magalhães
Juliana C. O. A. Ferreira
Marco Felipe Castro Silva
Mariana Ferriani
Ana Paula Sakamoto
Virginia Paes Leme Ferriani
Maraísa Centeville
Juliana Sato
Maria Carolina Santos
Eloisa Bonfá
Clovis Artur Silva
Panniculitis in childhood-onset systemic lupus erythematosus: a multicentric cohort study
Advances in Rheumatology
Lupus erythematosus panniculits
Childhood
Systemic lupus erythematosus and multicenter study
title Panniculitis in childhood-onset systemic lupus erythematosus: a multicentric cohort study
title_full Panniculitis in childhood-onset systemic lupus erythematosus: a multicentric cohort study
title_fullStr Panniculitis in childhood-onset systemic lupus erythematosus: a multicentric cohort study
title_full_unstemmed Panniculitis in childhood-onset systemic lupus erythematosus: a multicentric cohort study
title_short Panniculitis in childhood-onset systemic lupus erythematosus: a multicentric cohort study
title_sort panniculitis in childhood onset systemic lupus erythematosus a multicentric cohort study
topic Lupus erythematosus panniculits
Childhood
Systemic lupus erythematosus and multicenter study
url http://link.springer.com/article/10.1186/s42358-019-0049-9
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